XXX
DOB xxx
Condition: C1 Esterase Inhibitor Deficiency (AKA Hereditary Angioedema)
This is a rare blood condition causing swelling of soft tissue.
Cause of Attacks
Anything that kicks off the immune system, such as:
- Trauma, such as a heavy fall or bang
- Infection
- Hormones
Symptoms
Swelling of any soft tissue. Common swellings are:
- Starting with the hands and moving up the arms
- Starting with the feet and moving up the legs
- Genitals
- Abdomen
- Face and throat
An attack is often accompanied by a pale pink rash that looks like marble.
Treatment
XXX takes Tranexamic Acid daily, which controls the frequency & intensity of attacks. His dose will be increased in the week before a school trip.
He carries Berinert (C1 Esterase Concentrate) for administration during serious attacks. This is an intravenous medication, so can only be administered by trained medics (and his mum).
WHAT TO DO
Swelling of hands, feet, genitals is not life threatening. xxx will need to rest and the swelling will subside over 24/48 hours.
Swelling of the tummy is painful and will cause vomiting and diarrhea. To be on the safe side, take xxx to hospital and have them administer the Berinert.
*** Swelling of the face & throat can be life threatening, so he will need to go to the nearest hospital as a matter of urgency. Call an ambulance.
In France, emergency numbers are 15 or 112
If there is an emergency, there is a national hotline: 06.74.97.36.88
There is a hospital in Calais (Centre Hospitalier de Calais) and Albert (Centre hospitalier (Albert)), but they are general hospitals.
The nearest university hospital is Lille for Calais and Amiens for Albert.
In Belgium, the emergency number is 100.
Jan Yperman Ziekenhuis is the hospital in Leper.
During an Emergency / Hospital Visit
- Take the medication out of the cooler asap, as it must reach room temperature before it can be used.
- Make the medical staff aware of the condition as soon as possible. Give them the letter from the consultant.
He should be a priority and dealt with straight away without delay. Ensure that they understand this and react appropriately.
- xxx needs a cannula inserting in his vein.
- The medication needs mixing (xxx knows how to do this, should there be any confusion)
The medication should be given slowly over 5-10 mins.
xxx’s parents will be in the Country and will also be carrying extra Berinert.
xxx wears a Medicalert Bracelet. This states his condition and has a telephone number on it for emergency services to ring for information if needed.
Important numbers are:
Mother xxxx
Father xxx
Dr xxxx
Parent’s Itinerary
Frequently asked questions
1. Is there anything we can do to prevent an attack?
No. Although attacks can be triggered by a fall or infection, they can also happen for no reason at all.
2. Should xxx avoid physical activity?
Not at the moment. We monitor xxx’s reactions to sport closely and believe that unless he experiences recurring attacks after a certain activity, he should play as normal.
Bumps and falls are accidents and we treat them as such.
With rough games such as Rugby, we will be monitoring his reactions and will let you know if he needs to stop playing.
An exception to this would be boxing or other sports that involve deliberate hitting.
3. Does he take any medication?
Yes. Xxxx takes a tablet called Tranexamic Acid. He’s been on this since June 2009 when he started having recurring tummy swellings.
4. Does anyone else in the family have it?
Yes. This is a hereditary condition and Thomas’s dad suffers with it. The condition is not identical in each person and people often react differently. However, in our experience, Tom and his dad have similar reactions.
5. Is there a cure?
No.
6. Can it be passed on to other people?
Only from a parent, as it’s a genetic disorder.
7. Is it an allergy?
No. It’s nothing at all to do with allergies, although it can be confused with anaphylaxis. If the face swells it’s nothing to do with allergies and adrenaline (epi pens) will not work.
Other
European SOS 112
The number 112 can be dialled to reach emergency services - medical, fire and police - from anywhere in Europe. This Pan-European emergency number 112 can be called from any telephone (landline, pay phone or mobile cellular phone). Calls are free. It can be used for any life-threatening situation, including:
· Serious medical problems (accident, unconscious person, severe injuries, chest pain, seizure)
· Any type of fire (house, car)
· Life-threatening situations (crimes)
· SOS 112 website
· Information on the 112 number from the European Commission website